809 Primary orbital and adnexal non-Hodgkin lymphoma (POAL): A single centre study of 20 cases

Abstract

The clinical behaviour, the prognosis and the treatment of the POAL are subject to some controversies. We define POAL as a localised lymphoma in the orbital and adnexal structures (I E), lasting at least one month after a diagnosis and complete staging procedure used in NHL. We studied 20 patients (pts) with lymphoproliferative lesions, from 1974 to 1994, finding 14 with POAL (orbit 6; lachrymal gland 4; conjunctive 2; eyelids 2), 1 patient had bilateral lesions. For the recent pts we studied the immunophenotype the beta2 microglobulin, LDH, abdominal ecography or CT, orbital CT or MRI. We applied the up-dated Kiel Classification (1 high grade and 13 low grade, most of them lymphoplasmocytic or diffuse centrocytic type cells). They was 2.5% of our NHL. The median age was 68 years (range 16–87) and there were a female predominance (M/F 1: 2.5). No patients had monoclonal gammopathy or immune disorder. The initial presentation was ocular tumour or proptosis in all pts. Treatment was: (1) surgery 2 (1 relapsed, 1 maintained CR); (2) radiotherapy (RT): 9 (7 of them with doses of 30–40Gy, with the following late complications of RT (EORTC): G0=6, G1=3, G2=1; all pts but one attained CR; (3) RT plus chemotherapy (CH):3. Four of the 6 orbital lesions had elapsed (66.6%); 4 of them were treated only with RT. The 2 pts with conjunctive lesions have been treated with RT; 1 is in CR 24 months (m.) after treatment. Three of the 4 pts with lachrymal lymphoma had a disease free survival (DFS) of 5, 12 and 32m; one ofthem was treated only with surgery; in this group was the only pt with high grade lymphoma treated with success with RT only. One pt that refused further RT, died, with relapse, after radical surgical excision. The 2 pts with eyelid lesions were in DFS for more than 13 months. They were treated with RT. CH, as initial treatment after RT, has been done in one of our last pts that had lachrymal gland lymphoma because the MR after RT showed perhaps residual disease. We conclude that surgery, if done, must always be followed by local RT. Local RT provides a good and almost permanent local control of the disease. Doses of 30^_35 Gy controlled our low grade lymphomas with no late complications, when good shielding of the lens of the eye is possible. The prognosis for pts with small-cell lymphoma in the ocular adnexa seems difficult to predict, mainly for the orbit. The role of adjuvant Ch with CVP or CHOP-like regimen, after local RT need to be investigated for pts with bad prognosis factors as retroorbital lymphoma, bulky tumour and high grade malignancy histology.